Petropoulos AC, et al.

, J Neonatol Clin Pediatr 2019, 6: 038

DOI: 10.24966/NCP-878X/100038

HSOA Journal of
Neonatology and Clinical Pediatrics
Research Article

Frequency and Anatomical mal 4ch view RV, gives the primary clue leading to follow-up series
of scans that can determine the immediate post-natal anatomy and
Fetchers of Tricuspid Valve further management of the patient.
Conclusion
Atresia in Azerbaijan: Based Tv atr. can be accurately diagnosed prenatally. The key findings,
On Fetal Cardiology Findings differential diagnoses and management are discussed. Short review
of the literature of the disease is provided.
Keywords: Fetal Echocardiography; Prenatal diagnosis; Strategies
in treating tricuspid valve atresia; Tricuspid valve atresia
Petropoulos AC1,2*, Xudiyeva A3, Valiyeva Q4, Behbudov V3
and Ismaylova M3

Department of ICU (Cardiac, Neonatal, Pediatric), Great Ormond Street

1
Abbreviations
Hospital, London, UK

The “Aziz Aliyev” national postgraduate and CME medical training center,
2 Tv atr.: Tricuspid Valve Atresia
Baku, Azerbaijan 4ch: Four Chamber
CHD: Congenital Heart Defect
3
Department of Pediatric Cardiology, Merkezi Klinika, Baku, Azerbaijan
HRHS: Hypoplastic Right Heart Syndrome
Department of Pediatrics, the National Ophthalmology Center, Baku,
4
HLHS: Hypoplastic Left Heart Syndrome
Azerbaijan
RV: Right Ventricular
RA: Right Atrium
Tv: Tricuspid Valve
Abstract d-TGA: D- Transposition of Great Arteries
Aim ToF: Tetralogy of Fallot
LA: Left Atrium
To describe the incidence, the prenatal diagnosis and the strategy
of treatment of patients suffering from Tricuspid Valve Atresia (Tv
ASD II: Secundum type Atrial Septal Defect
atr.). PFO: Paten Foramen Obale
AVv: Atrioventricular Valve
Methods
Mv: Mitral Valve
We describe 10 cases of prenatal diagnosis from our series of
VSD: Ventricular Septal Defect
our fetal cardiac clinic in three different hospitals, during the period
2012-18 from Baku-Azerbaijan. We review the existing literature on CoA: Aortic Coarctation
the pre-natal diagnosis and post-natal management. PDA:Patent Ductus Arteriosus
Results
GA’s: Great Arteries
TA: Trancus arteriosus
We present the key findings for diagnosis by fetal echocardiog-
DORV: Double Outlet RV
raphy that include: Demonstration of no patent tricuspid valve on
the 4-Chamber (4-ch) view, no flow across the Tv on pulse or color DOLV: Double Outlet LV
Doppler flow mapping, small Right Ventricle (RV) with associated PAvS: Pulmonary valval stenosis
Ventricular Septal Defect (VSD) and the Great Vessels (GA’s) con- Qp /Qs: Pulmonary-to-Systemic blood flow ratio
nectivity to the ventricles (normal related or transposed). The abnor-
LVvo: LV Volume Overloading
SVD: Spontaneous Vaginal Delivery
*Corresponding author: Petropoulos AC, Department of ICU (Cardiac, Neona-
tal, Pediatric), Great Ormond Street Hospital, London, UK, Tel: +44 7444251425; PGE: Alprostadil
E-mail: Andreas.Petropoulos@gosh.nhs.uk BT-Shunt: Blalock-Taussig Shunt
Citation: Petropoulos AC, Xudiyeva A, Valiyeva Q, Behbudov V, Ismaylova M TCPC: Total Cavopulmonary Anastomosis
(2019) Frequency and Anatomical Fetchers of Tricuspid Valve Atresia in Azerbai-
jan: Based On Fetal Cardiology Findings. J Neonatol Clin Pediatr 6: 038. Interdiction
Received: November 04, 2019; Accepted: November 25, 2019; Published: Tricuspid Valve Atresia (Tv atr.) is a form of a Hypoplastic Right
December 2, 2019 Heart Syndrome (HRHS). The defect was firstly published in 1817
Copyright: © 2019 Petropoulos AC, et al. This is an open-access article dis- by Kreysig. The essence of the malformation lies in the absence of
tributed under the terms of the Creative Commons Attribution License, which a functional tricuspid valve between the Right Atrial (RA) and the
permits unrestricted use, distribution, and reproduction in any medium, provided Right Ventricle (RV) [1].
the original author and source are credited.
Citation: Petropoulos AC, Xudiyeva A, Valiyeva Q, Behbudov V, Ismaylova M (2019) Frequency and Anatomical Fetchers of Tricuspid Valve Atresia in Azerbai-
jan: Based On Fetal Cardiology Findings. J Neonatol Clin Pediatr 6: 038.

• Page 2 of 7 •

Epidemiology and simulate sub-aortic obstruction in patients with transposition of

the GA’s can be found [6]. GA’s relationship is variable and forms
In our series, Tv atr., is described as the third most common cy- the basis of classification [7]. Aorta is either normal or slightly larger.
anotic Congenital Heart Defect (CHD). From 774 fetal scans, (Pet- Obstruction to the pulmonary outflow tract is frequent [7].
ropoulos AC et al., unpublished data), was the second most common,
after Tetralogy of Fallot (ToF) [2]. The other 2 frequently observed
were: TGA and DORV. Worldwide prevalence is reported between
1- 2.3% of all CHD [2] (Table 1). Our incidence was 2.7 times higher
than the reported worldwide. We believe that the spectrum of HRHS
is higher in our area due to a possible endogenous genotype like those
reported up to date [3]. This can probable be explained as in Azer-
baijan the incidence of CHD detected among term neonates in the
country is high 1.8% [4].

Figure 1: Anatomical Variations of Atretic Tricuspid Valve.

Table 1: Geographic Prevalence of Tv atr.

Anatomical Features
Most common histological type of Tv atr., is the muscular, found
in 89% of cases. Localized fibrous thickening in the site of the Tv
[5] can be found (Figure 1). Rare types are: membranous (6.6%), in
which the atrioventricular part of the membranous septum forming
the floor of the Right Atrium (RA) is atretic. This type is associat-
ed with absent PAv leaflets. In 2.6% of cases, an Ebstein type, com-
Figure 2: Most common types of Tv atr.
bining the essence of both defects can exist. Approximately 1% the
valval cusps are minute and fused. In the atrioventricular canal type,
(0.2%) an atretic leaflet of the common Atrioventricular Valve (AVv),
In 30% of patients, various associated cardiac defects are present,
guards the inflow to the RV [5]. In the unguarded type (0.6%), the
with aortic Coarctation (CoA) and persistent left superior vena cava,
atrioventricular junction is unguarded, but the inlet component of the
most common. These can be:
RV is separated from its outlet by a muscular shelf [5] (Figure 4). In
an atretic Tv setting, an intra atrial communication to drain systemic • Interrupted aortic arch.
venous return to the Left Atrium (LA) is obligatory. This can either • Absent PAv.
be an unrestrictive secundum type Atrial Septal Defect (ASD II) or
• Aneurysm of the atrial septum.
even a large or restrictive Paten Foramen Obale (PFO). This will lead
• Aorto-Pulmonary fistula.
to volume overload and finally, hypertrophy of the LV and MvR+. Its
severity will correlate with any delay in surgical repair. An obligato- • Right aortic arch.
ry- in the classic form -VSD is present allowing the blood to enter the • Anomalous origin of the coronary arteries from the pulmonary ar-
hypoplastic RV that size and function very, regarded to the relation- tery (ALCAPA).
ship of the GA’s [5]. The VSD may be: Cono-ventricular, perimem- • Anomalous origin of the left and or right subclavian artery.
branus, cono-septal mal-aligned, muscular or atrioventricular canal • Common atrium.
type [5]. From these the muscular is most frequent and can restrict by • Cor triatriatum dexter type.
time. Subpulmonary stenos is in patients with normally related GA’s • Coronary sinus atrial septal defect.

Volume 6 • Issue 3 • 100038

J Neonatol Clin Pediatr ISSN: 2378-878X, Open Access Journal
DOI: 10.24966/NCP-878X/100038
Citation: Petropoulos AC, Xudiyeva A, Valiyeva Q, Behbudov V, Ismaylova M (2019) Frequency and Anatomical Fetchers of Tricuspid Valve Atresia in Azerbai-
jan: Based On Fetal Cardiology Findings. J Neonatol Clin Pediatr 6: 038.
• Double aortic arch.
• Hemi and persistent Truncus Arteriosus [8].
• Hypoplastic ascending aorta and/or aortic.
• Atresia Primum (ASD).
• Uhl’s heart.
• Patent Ductus Arteriosus (PDA).
• Total anomalous pulmonary venous drainage.
• Tubular hypoplasia of the aortic arch.
• Aortic valve stenosis.
• Isomerism atrial appendages syndromes.
• Anomalous drainage of coronary sinus into LA [9] Associated ex-
tra cardiac congenital malformations are reported in 22%. Most
common: Chromosomal anomalies with frequent VACTERL as-
sociation, unilateral renal agenesis, hypospadias, hydrothorax,
megacystis and agenesis of the ductus venosus [10,11].
Classification of the Defect
Many classifications exist. The most commonly used is based on
the interrelations of the GA’s and the amount of PAv Stenosis (PAvS)
that may exist [7].
• Type I Normally related GA’s (~70%) (Figure 2).
• Type II D-Transposition of the GA’s (~29%) (Figure 2).
• Type III GA’s positional abnormalities other than D-transposition:
a. Subtype-1: L-transposition, b. subtype-2: DORV, c. subtype-3:
DOLV, d. subtype-4: D-malposition of GA’s, e. subtype-5: L-mal-
position of GA’s.
• Type IV TA [7,10].
• Subgroup a: With PAv atresia.
• Subgroup b: With PAvS or hypoplasia.
• Subgroup c: Without PAvS.
Further, the status of VSD-patent or not-and the presence of other
associated malformations are described.
This unified classification [7] includes all types described by pre-
vious classifications by Kuhne, Edwards and Burchell, and by Keith,
Rowe and Vlad [12].
Embryology
The AVv’s develop shortly after the atrioventricular cushions are
activated. The anterior and posterior Tv leaflets develop by undermin-
ing of a skirt of ventricular muscle tissue. The septal leaflet mostly
develops from the inferior endocardial with a small contribution from
the superior cushion. The process of undermining extends until the
AVv junction is reached. Resorption of the muscle tissue produces
normal-appearing valve leaflets and hordae tendineae. Fusion of de-
veloping valve leaflet components results in stenosis or atresia [13].
Whether a muscular type or a fused valve develops depends on the
embryologic stage in which aberration takes place [6,14] (Figure 1).
The embryologic, pathologic, clinical, and imaging features of Tv
stenosis and/or atresia are similar. This leads rare seen Tv stenosis, to
be calcified in this group of CHD [14,15].
Pathophysiology
During the fetal period, systemic venous blood return is forced
across the PFO into the left heart. The lowered PaO2 to the brain and
J Neonatol Clin Pediatr ISSN: 2378-878X, Open Access Journal
DOI: 10.24966/NCP-878X/100038
• Page 3 of 7 •
heart and elevated PaO2 to the lungs do not produce postnatal abnor-
malities [7]. In patients with type I and associated PAv atresia types
Ia and IIa, their pulmonary blood flow is supplied entirely through
the PDA. This shunt delivers 8-10% of combined ventricular output
compared with 66% of combined ventricular output in a normally
developed fetus. Acute angulations of the PDA occurs, because of
reversed direction of DA flow. These two factors make the PDA less
responsive to postnatal stimuli than usual.
In a fetus with type I anatomy and a small or absent VSD, almost
all LV output is ejected into the aorta and transported to the placenta.
Therefore, as the aortic isthmus carries a larger-than-normal cardiac
output CoA is rarely found in this subtype.
Fetuses with type II have increased portion of the blood entering
by DA into the descending aorta. This results minimal flow across the
aortic isthmus. As a result, this can lead to frequent seen CoA [1,16].
Postnatally, the obligatory PFO/ASDII causes a mixing of system-
ic and pulmonary venous returns, in the LA and passes into the LV [6-
7,16]. This flow pattern occurs in all types of Tv atr. except of type III
and subtypes 1 and 5. In these exceptions, the atretic Tv is left sided
due to ventricular inversion; therefore, the pathophysiology is that of
Mv atresia with consequent left-to-right shunting of pulmonary ve-
nous return [6,16].
In patients with normally related GA’s, and a VSD, shunting
through the VSD permits perfusion of the lungs. When the VSD is ab-
sent, pulmonary blood flow is derived by the PDA or aorto-pulmonary
collateral vessels [6-7,16].
In patients with D-TGA’s, the lungs receive blood flow from the
LV. The aorta receives blood from the LV via the VSD and RV [6-
7,16]. In other types, the routes of aortic and pulmonary artery flow
depend on the size of the VSD and associated cardiac defects.
Additional conditions that affect the clinical presentation and
treatment plan are:
Arterial desaturation
An amount of systemic desaturation is present in all patients, due
to obligatory mixture of systemic, coronary, and pulmonary venous
returns, in LA. The degree of arterial desaturation depends on the
amount of pulmonary blood- flow [7,16]. The arterial oxygen satu-
ration has a curvilinear relationship, with a pulmonary-to-systemic
blood flow ratio (Qp /Qs) that reflects the pulmonary blood flow. Qp/
Qs ratio of 1.5-2.0 results in adequate oxygen saturation. Higher Qp
does not significantly increase saturation and produces LVvo and pul-
monary over-circulation, with tachypnoea that can lead to CHD and
pulmonary edema [7,16].
Pulmonary blood flow
Clinically, patients depend mainly on the quantity of Qp [7,16].
Neonates with markedly decreased Qp are likely to present early with
severe cyanosis, hypoxemia, acidosis. When Qp is increased, the ne-
onates may appear mild cyanotic but show signs of heart failure lat-
er. Patients with pulmonary oligemia generally have type I disease.
Those with pulmonary plethora usually have type II and, rarely, type
I c [7,16].
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Citation: Petropoulos AC, Xudiyeva A, Valiyeva Q, Behbudov V, Ismaylova M (2019) Frequency and Anatomical Fetchers of Tricuspid Valve Atresia in Azerbai-
jan: Based On Fetal Cardiology Findings. J Neonatol Clin Pediatr 6: 038.
In patients with a type I defect, the obstruction can be valvar, sub
valvar, or frequently, at the VSD level. In patients with a type II de-
fect, the obstruction is valvar or sub valvar. In patients with a type I
defect, with large VSD, without PAvS, the Qp is inversely proportion-
al to the pulmonary-to-systemic vascular resistance ratio. If a PDA or
a surgical systemic-to-pulmonary artery shunt has performed, Qp, is
proportional to the size of the natural or surgical shunt [7,16].
LV volume overloading (LVvo)
LV ejects the entire outputs. Therefore, LVvo is present in all pa-
tients [6,16]. The degree of LVvo increases further if mild or absent
pulmonary outflow obstruction is noted or if systemic-to-pulmonary
artery shunting was performed. Maintaining normal LV function is
essential for a successful Fontan operation. LV function tends to de-
crease as age increases, decreasing Qp/Qs, and arterial desaturation
[16,17].
Obstruction of the interatrial communication
PFO/ASD II, is essential for survival. As entire systemic venous
blood must pass through, any obstruction if present can be clinically
significant [6,16]. PFO/ASD II obstruction, is present when the mean
pressure difference between RA-LA is more than 5 mm Hg [1,7] and/
or the diameter of the defect is less than 5 mm [1,7].
Findings
In our series we detected ten cases of Tv atr. These were six of
type I from which four was subgroup b. One was subgroup a and one
subgroup c. Three cases were type II. Two were subgroup b and one
subgroup c. Finally, one case was type III - subtype-2 and subgroup
b. So, it could be also called DORV with Tv atr. and PAv st. The indi-
cations for fetal cardiology evaluation were: 1. Inbreeding (first- and
second-degree relatives among parents) 3/10 (30%) 2. Abnormal car-
diac screening examination during the 20th week anomaly scans 2/10
(20%) 3. IVF conceived fetuses, 2/10 (20%), 4. First-degree relative
of a fetus with congenital heart disease1/10 (10%), 6. Combination of
maternal age and increased nuchal translucency 1/10(10%) 7. Mater-
nal Metabolic disorder (Diabetes Mellitus) 1/10(10%).
The initial scans were done mostly after the 20th week of gesta-
tion, with a mean gestational age (21+/-3 weeks). The assessment
was done using color Doppler -2D echocardiography, by a General
Electric Vivid 7 device, with an appropriate cardiac fetal probe and
software program.
The findings were verified with echocardiography, cardiac cathe-
terization, cardiac CT and MRI studies were indicated, post-natal, and
postmortem examination in pregnancies that were interrupted.
Post-natal 4 female and 2 male neonates were delivered by ce-
sarean section between 36-38th week of gestation in centers that had
Neonatal Intensive Care Units. Their anatomy was in all cases type I.
Four were subgroup b and the other two were subgroup’s a and c.
All families were thoroughly consulted on two consecutive meet-
ings on findings and further management and outcomes that could be
offered locally or abroad.
From the ten cases four 4/10(40%) choose to terminate the preg-
nancy. These were 3/4 (75%) type II and one (25%) was type III - sub-
type-2 and subgroup b.
J Neonatol Clin Pediatr ISSN: 2378-878X, Open Access Journal
DOI: 10.24966/NCP-878X/100038
• Page 4 of 7 •
The interruption of pregnancy took place between 24 +/-2 weeks
of gestation. Interruption of pregnancy due to significant medical
issues in legal in Azerbaijan before the 28th week of gestation. The
post-mortem revealed two females’ fetuses and two males. No macro-
scopic additional malformations were noticed. The cardiac anatomy
was identical to the prenatal findings.
From the delivered babies four received prostin during the first
day of life and was later offered an early Blalock-Taussig shunt at age
50+/-6 days old. One type I-subgroup C presented at age 35 days old
with symptoms of CHF. She was palliated with a band of her main
Pulmonary artery.
One female type I-subgroup b had balanced physiology and did
not require any initial stage of palliative surgery or prostin [1].
Discussion
Tv atr. is a rather rare prenatal diagnosed CHD. In our series it was
the third most common cyanotic CHD [2]. Probable this was due to
the cumulation of possible pathological cases as we are the referral
center for fetal cardiology in the country. The true incidence of the
defect will be determined only by a prospective epidemiology study
among living neonates [4].
Tv atr. as a defect in the spectrum of HRHS must be linked to an
abnormal 4-chamber view and therefore, it must be potentially detect-
able during routine obstetric sonographic scans [18].
Fetal Cardiology diagnosis must emphasize on:
1. No patency of Tv. This can be seen by 2D and color Doppler (Fig-
ure 3) [11,19].
2. Cardiac axis, position, cardiothoracic ratio and situs of the viscera
are normal.
3. Discrepancy between RV and LV is always present.
4. MvR+ is frequent and due to LVvo. [19] (Figure 4).
5. Nearby 70% have normal related GA’s, frequently with asymme-
try in the PAv/Aov ratio. Approximately 29% have D-TGA [11]
(Figure 2).
6. Aortic arch in most cases is normal. The ductal arch is larger than
normally, with a reversed ductal flow [19].
7. Moderate at least size VSD with a left to right shunt is frequently
present [11] (Figure 4).
8. Rarely restrictive right to left flow through the FO [19].
9. The LV has a normal function and no evidence of endocardial fibro
elastosis is seen in undeveloped RV [17,19].
Figure 3: a) absence of color flow mapping through the atretic tricuspid valve and
regurgitate jet through the dilated Left Ventricular; b) Fetal echocardiography, colored
Doppler showing no patency of the atretic tricuspid valve b) Fetal echocardiography
showing c) Fetal echocardiography showing common type I tricuspid valve atresia.
Volume 6 • Issue 3 • 100038
Citation: Petropoulos AC, Xudiyeva A, Valiyeva Q, Behbudov V, Ismaylova M (2019) Frequency and Anatomical Fetchers of Tricuspid Valve Atresia in Azerbai-
jan: Based On Fetal Cardiology Findings. J Neonatol Clin Pediatr 6: 038.

• Page 5 of 7 •

alprostadil (PGE1). PGE1 is effective only in the neonatal period. A

modified or classical Blalock-Taussig Shunt (BT-Shunt) should be of-
fered to improve cyanosis.

If congestive heart failure is present, diuretics and after load-re-

ducing agents as Angiotensin-converting enzyme inhibitors may be
administered to reduce after load and augment LV function, especial-
ly after a bidirectional Glenn procedure and a Fontan type operation.
Anticoagulants and platelet-inhibiting drugs are useful in preventing
thrombus formation after a Glenn or a Fontan completion [20,27].
The American Heart Association recommends that prophylaxis
with antibiotics be given before any bacteremia-producing proce-
dures are performed [20,27].

Surgical Treatment
Figure 4: Fetal echocardiography showing VSD setting, hypoplastic RV in the setting
of tricuspid valve atresia.
The patient’s age, weight, anatomic and physiologic status deter-
mines the types of surgery recommended. The overall objective is
to achieve a Fontan circulation by a total cavopulmonary connection
[20].
When detected prenatally, a 4-week follow-up during the preg-
nancy is suggested to verify the final anatomy. As extra cardiac mal- In neonates and young infants with pulmonary oligemia, as a first
formations and chromosomal abnormalities is low amniocentesis is step, a classic or modified BT-shunt is undertaken to improve oxygen-
not recommended [18]. The risk of recurrence is only 1-2% in a next ation [27].
pregnancy [18,20]. Most are sporadic although familial cases have
been reported [21]. In patients aged 6 months to 2 years, a bidirectional Glenn proce-
dure (Figure 5) is the goal standard approach. For patients older than
If no prenatal data exist, postnatal diagnosis can be achieved by 2 years, total cavopulmonary connection may be performed, but most
the failing pulse oximetry screening test [22,23]. The clinical presen- authorities suggest staging by using an initial bidirectional Glenn op-
tation will be based on the existing anatomy and physiology. If oxy- eration followed by Fontan completion after 6-12 months, or even
gen saturation is less than 70-75%, an intervention to improve pulmo- after the age of 4 years old [20,27].
nary oligemia must be considered. Electrocardiogram is diagnostic,
revealing RA dilatation, abnormal superiorly oriented QRS axis,
LVH, and decreased RV forces. RA dilatation, with tall and peaked P
waves (≥2.5 mm) in lead D-II and right chest leads are present in 75%
of patients. The so-called P-tricuspidale with a double peak, spike
and dome configuration may be present. Abnormal vector is present
in 80% of patients with Tv atr., type I but only in 50% of patients with
type II or III [24]. Echo-2D will establish the diagnosis and verify
all the pathology, in most of the cases. Reveals a small RV and an
enlarged RA, LA and LV. In commonest muscular type, a dense band
of echoes is observed in the Tv location. The anterior leaflet of the
detectable AVv is attached to the left side of the interatrial septum.
These features are best demonstrated in the apical and subcostal 4-ch
views. Imaging of ASD and VSD are essential. CoA, more frequent
in patients with type II, will be showed in the suprasternal view [25].
Figure 5: Surgical illustration of a Bi- Directional glenn anastomosis.
Cardiac catheterization that was so commonly used, in the era of
sophisticated Echo-2D and cardiac MRI is nowadays, limited. A few
cases in which specific hemodynamic data are required, verification
During bidirectional Glenn surgery, any narrowing of the pul-
of some of the Choussat criteria for planning surgical repair or a com-
bination of diagnostic and interventional procedures when needed, monary artery should be repaired. Other additional existing lesions
will enter the Cath Lab [20]. An existing CoA can be addressed ini- such as sub-aortic obstruction and MvR+ should also be addressed
tially by an interventional procedure [2,22,26-27]. Finally, interven- [20,27]. Before Fontan completion, especially in delayed presenting
tional procedures can be used following the surgical repair to address or poorly followed up cases, some suggest cardiac catheterization to
residual conditions. be undertaken. This approach is widely abandoned as MRI studies
become more frequently used. A cardiac catheterization or an MRI
Medicine Treatment study needs to investigate the possibility of existing aorto-pulmonary
Neonates with severe cyanosis, pulmonary oligemia and PDA-de- collaterals. If collateral vessels are present, they should be occluded
pendent pulmonary flow can benefit by intravenous administration of with coils prior to the Fontan Completion [20,26-27].

Volume 6 • Issue 3 • 100038

J Neonatol Clin Pediatr ISSN: 2378-878X, Open Access Journal
DOI: 10.24966/NCP-878X/100038
Citation: Petropoulos AC, Xudiyeva A, Valiyeva Q, Behbudov V, Ismaylova M (2019) Frequency and Anatomical Fetchers of Tricuspid Valve Atresia in Azerbai-
jan: Based On Fetal Cardiology Findings. J Neonatol Clin Pediatr 6: 038.
Most surgeons currently prefer extra-cardiac conduit diversion of
inferior vena cava blood into the right pulmonary artery. To address
the growth issue related to extra-cardiac Fontan surgery, some sur-
geons use autologous or bovine pericardial roll grafts [20,27].
In patients with D-TGA’s, early banding of the PA, relief of pos-
sible CoA and bypassing using a Daimus-Kaye- Stansel procedure
and/or resecting if technically possible of the sub-aortic obstruction
should be incorporated into the management plan [20,27,28].
Following the Fontan completion, immediate, mid and long-term
complications that can occur are: Persistent pleural effusions, neuro-
logic complications such as strokes or brain abscesses, arrhythmias,
thromboembolic events, obstruction at the anastomotic sites or in the
pulmonary artery, residual shunts and/or systemic venous congestion
presenting as protein-losing enteropathy and rarely plastic bronchitis.
All these need to be aggressively treated to sustain a New York Heart
Association stage I to II and improve quality and life expectancy [20].
In patients with “Failing Fontan” after excluding/addressing ob-
structions, residual shunts and arrhythmias, apart from conventional
treatment, consideration for resynchronization by pacing the RA and
LV and conversion of atrioventricular Fontan’s to Total Cavopulmo-
nary Anastomosis (TCPC) (Figure 6) Cardiac transplantation should
be considered as the last step of further treatment in a failing Fontan
circulation [20,27,29].
Figure 6: Fontan Circulation after TCPC in palliation of Tv atresia.
Prognosis
Prognosis in untreated Tv atr. is poor. Early identification, rapid
and safe transport to a pediatric cardiology center, noninvasive di-
agnosis, availability of PGE1 to keep PDA, and recent advances in
anesthesia and surgical techniques have improved the prognosis of
these babies. Complications associated with the classic Fontan are
decreased with the widespread use of staged TCPC (Figure 6) [20,27-
28].
Conclusion
Tv atr. is a complex cyanotic critical CHD requiring multiple med-
ical, intervention and surgical treatment. Prenatal diagnosis is avail-
able alternating early morbidity and mortality but also offers to parent
the alternative of an elective abortion. A detailed explanation of the
cardiac defect and treatment required should be given to the parents
J Neonatol Clin Pediatr ISSN: 2378-878X, Open Access Journal
DOI: 10.24966/NCP-878X/100038
• Page 6 of 7 •
at the time of diagnosis and repeated as needed [20,27]. Further, pulse
oximetry screening after the 3ed postnatal day can detect this CHD
[22].
Conflict of Interest
None of the authors have any conflict of interest in this series of Tv
atr.
References
1. Petropoulos AC, Xudiyeva A, Valiyeva Q, Behbudov V, Ismaylova M
(2018) Prenatal diagnosis and management of tricuspid valve atresia: A
case report and review of literature. J Neonatol Clin Pediatr 5: 1-7.
2. Petropoulos AC, Xudiyeva A, Valiyeva Q, Behbudov V, Maharammova
T, et al. (2015) Incidence of congenital cardiac disease, detected by fe-
tal echocardiography, in azerbaijan. experience of three centers during a
thirty month period. Card in the Young 25: 1.
3. Dimopoulos A, Sisko RJ, Kay DM, Rigler SL, Druschel CM, et al.
(2017) Rare copy number variants in a population based investigation of
hypoplastic right heart syndrome. Birth Defects Res109: 8-15.
4. Petropoulos AC, Hudiyeva A, Behbudov V, Mustafayeva G, Guliyev N,
et al. (2019) The Incidence of Congenital Heart Disease in Baku-Azer-
baijan. Prospective Epidemiology Study. Arch Dis Child-epa 104: 1-428.
5. Rao PS (1992) Terminology: Is tricuspid atresia the correct term to use?
In: Rao PS (eds.). Tricuspid Atresia, (2nd edn). Futura, Mount Kisco, NY.
Pg no: 3-15.
6. Ottenkamp J, Wenink AC, Quaegebeur JM, Rohmer J, Gittenberger-de
Groot AC, et al. (1985) Tricuspid atresia. Morphology of the outlet
chamber with special emphasis on surgical implications. J Thorac Car-
diovasc Surg 89: 597-603.
7. Rao PS (1990) Tricuspid atresia. In: Long WA (eds.) Fetal and Neona-
tal Cardiology. WB Saunders, Philadelphia, Pennsylvania, USA. Pg no:
525-40.
8. Rudolph AM (1974) Tricuspid atresia with hypoplastic right ventricle.
Congenital Disease of the Heart. Year Book Medical, Chicago, Illinois,
USA. Pg no: 429-61
9. Rao PS, Levy JM, Nikicicz E, Gilbert-Barness EF (1991) Tricuspid
atresia: Association with persistent truncus arteriosus. Am Heart J 122:
829-835.
10. Rao PS, Covitz W, Chopra PS (1992) Principles of palliative manage-
ment of patients with tricuspid atresia. In: Rao PS (eds.). Tricuspid Atre-
sia, (2nd eds.). Futura, Mount Kisco, NY, USA. Pg no: 297-320.
11. Berg C, Lachmann R, Kaiser C, Koiowski P, Stressig R, et al. (2010)
Prenatal diagnosis of tricuspid atresia: Intrauterine course and outcome.
Ultrasound Obstet Gynecol 35: 183-190.
12. Rao PS (1980) A unified classification for tricuspid atresia. Am Heart J
99: 799-804.
13. Van Mierop LH, Gessner IH (1972) Pathogenetic mechanisms in con-
genital cardiovascular malformations. Prog Cardiovasc Dis 15: 67-85.
14. Keefe JF, Wolk MJ, Levine HJ (1970) Isolated tricuspid valvular steno-
sis. Am J Cardiol 25: 252-257.
15. Lang D, Oberhoffer R, Cook A, Sharland G, Allan L, et al. (1991) Patho-
logic Spectrum of Malformations of the Tricuspid Valve in Prenatal and
Neonatal Life. J Am Coll Cardiol 17: 1161-1167.
16. Rao PS (1991) Perinatal circulatory physiology. Indian J Pediatr 58: 441-
451.
Volume 6 • Issue 3 • 100038
Citation: Petropoulos AC, Xudiyeva A, Valiyeva Q, Behbudov V, Ismaylova M (2019) Frequency and Anatomical Fetchers of Tricuspid Valve Atresia in Azerbai-
jan: Based On Fetal Cardiology Findings. J Neonatol Clin Pediatr 6: 038.
17. Graham TP Jr, Erath HG Jr, Boucek RJ Jr, Boerth RC (1980) Left ven-
tricular function in cyanotic congenital heart disease. Am J Cardiol 45:
1231-1236.
18. Tongsong T, Sittiwangkul R, Wanapirak C, Chanprapaph P (2004) Pre-
natal diagnosis of isolated Tricuspid valve atresia: Report of 4 cases and
review of the literature, J Ultrasound Med 23: 945-950.
19. Bader RS, Hornberger LK, Huhta JC (2008) Tricuspid Atresia in The
Perinatal Cardiology Handbook. Mobile Medicine Series, USA.
20. Kanakis MA, Petropoulos AC, Mitropoulos FA (2009) Fontan operation.
Hellenic J Cardiol 50: 133-141.
21. Tzifa A, Gauvreau K, Geggel RL (2007) Factors associated with devel-
opment of atrial septal restriction in patients with tricuspid atresia involv-
ing the right-sided atrioventricular valve. Am Heart J 154: 1235-1241.
22. Petropoulos AC (2018) Right use of Pulse Oximetry must be used as a
Screening Test for early detection of critical Congenital Heard Diseases.
Open J Cardiol Heart Dis 1-3.
23. Mahle WT, Newburger JW, Matherne GP, Smith FC, Hoke TR, et al.
(2009) Role of Pulse Oximetry in Examining Newborns for Congenital
Heart Disease: A Scientific Statement from the AHA and AAP. Pediatric
124: 823-836.
J Neonatol Clin Pediatr ISSN: 2378-878X, Open Access Journal
DOI: 10.24966/NCP-878X/100038
• Page 7 of 7 •
24. Gamboa R, Gersony WM, Nadas A (1996) The Electrocardiogram in
Tricuspid Atresia and Pulmonary Atresia with Intact Ventricular Septum.
Circulation 34: 24-37.
25. Covitz W, Rao PS (1992) Non-invasive evaluation of patients with tri-
cuspid atresia ((roentgenography, echocardiography and nuclear angi-
ography). In: Rao PS (ed.) Tricuspid Atresia, (2nd edn). Futura, Mount
Kisco, NY. Pg no: 165-82.
26. Rao PS (1992) Cardiac catheterization in tricuspid atresia. In: Rao PS
(ed.) Tricuspid Atresia, (2nd edn). Futura, Mount Kisco, NY. Pg no: 153-
78.
27. Mavroudis C, Backer CL (2003) Pediatric cardiac Surgery, 3rd ed., Phil-
adelphia, Pennsylvania, USA.
28. Rao PS (2007) Principles of management of the neonate with congenital
heart disease. Neonatology today 2: 1-10.
29. Jayakumar KA, Addonizio LJ, Kichuk-Chrisant MR, Galantowicz ME,
Lamour JM, et al. (2004) Cardiac transplantation after the Fontan or
Glenn procedure. J Am Coll Cardiol 44: 2065-2072.
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